ectrodactyly

After the operation, a small, light scar remains, which is almost invisible with appropriate rehabilitation treatment.

pathology

Experienced hand surgeons perform the full spectrum of reconstructive surgery on children and adults with congenital and acquired pathologies of the hand and the entire upper extremity.

The hand is one of the most important parts of our body. In young patients it is also a tool for active learning and intellectual development. The sooner the full function of the hand is restored, the more actively the child will develop.

Hand surgery is a separate branch of surgery that deals directly with the reconstruction and restoration of hand function.

The hand has complex anatomy and enables highly coordinated movements that are essential for a fulfilling life.

Congenital hand pathology in children is a specialty of hand surgery that requires a thorough knowledge of the child's physique, the variety of congenital pathologies and the peculiarities of surgical interventions in children.

The specialists of our clinic have many years of experience in reconstructive surgery for children with congenital and acquired hand pathologies. Anesthesia support makes it possible to perform complex hand operations on children without 'harmful' drugs, thanks to regional blocks. In a very short time after the operation, the child can return to his normal rhythm of life without any health risks.

The hospital is equipped with comfortable wards and the most modern equipment from top European companies.

Clinodactyly of the fingers

Clinodactyly is a disorder in which the phalanges of the fingers are bent at an angle to the axis of the finger. It can be congenital (with an extra phalanx) or acquired (as a result of trauma).

Congenital clinodactyly of the first and fifth toes is the most common.

A slight deviation of the phalanx (up to 10 degrees) is quite common and does not require surgical intervention. Of course, minor angular misalignments of the finger can also be corrected if a better overall aesthetic appearance is desired.

Congenital clinodactyly with a deformation angle of more than 20 degrees requires surgical correction to improve hand function and eliminate the cosmetic defect.

Clinodactyly of the little finger is a common malformation, but in most cases it is only a small cosmetic defect that only needs to be corrected if the aesthetic appearance of the hand is to be improved. The treatment methods we use allow us to preserve the function of the adjacent joints and shorten the rehabilitation time. Clinodactyly must be corrected to improve both the functional and cosmetic condition of the hand.

Manifestation of EEC syndrome (core syndrome)

Ectrodactyly is a cleft hand and/or foot with the absence of one or more middle fingers, also known as cleft hand and foot malformation (SHFM). The appearance of the hands and feet is often compared to the claws of a crab. It often occurs in conjunction with syndactyly (fusion of the fingers).

Ectodermal dysplasia is manifested by structural abnormalities in all ectodermal derivatives. The lesions affect hair, teeth and nails (11). Their hair and eyelashes are dull, sparse and stiff. It is characterized by dental abnormalities, hypoplasia of tooth enamel, and reduced pigmentation of hair, skin, and iris, resulting in a lighter color. The syndrome is also often associated with photophobia [1], nasolacrimal duct obstruction [12] and conductive hearing loss [13].

Ectodermal dysplasia is characterized by the absence of sweat glands, which is manifested by a hypohidrotic syndrome with severe dryness and scaly skin. The characteristic symptom of this syndrome is a hoarse, hoarse voice due to impaired vocal cord hydration, leading to incomplete vocal cord closure (14).

The speech disorder in patients with EEC syndrome has several causes. First, it is due to the consequences of cleft lip and/or palate and hyperacusis; Secondly, not only the changed vocal cords, but also dental diseases can hinder proper articulation. Third, hearing loss due to ossicular abnormalities often progresses and leads to cognitive and language impairments.

Children with this syndrome usually have a normal level of intelligence. Treatment consists of correcting deformities of the face and limbs. The symptoms of ectodermal dysplasia appear in the postnatal period (1). More than half of all patients with EEC syndrome have congenital malformations (malformations) of the urinary tract. These include megaureters, hydronephrosis, ureterocele, renal aplasia, genital anomalies, and ureteral reflux with frequent urinary tract infections (12).

Prenatal diagnosis of EEC syndrome

Given the severity of EEC syndrome, a prenatal diagnosis is quite possible. However, to date, few publications on the diagnosis of this rare syndrome have been found in the world literature [15-17], which is probably due to the rarity of this anomaly. Generally, the time frame for diagnosis is 16-30 weeks of pregnancy. The use of new 3D/4D ultrasound technology with surface reconstruction techniques is very helpful in the diagnosis of this pathology given the pronounced changes in the phenotype of the face and limbs (16).

The first description of the prenatal diagnosis of the syndrome dates back to 1993 and goes back to M. Bronshtein and R. Gershoni, when transvaginal echography revealed cleft lip and palate and ectrodactyly in the 14th week. This fact is unique in that it is believed to be the first known case of a congenital anomaly discovered prenatally in the first trimester. It is also noteworthy that the authors not only describe separately identified fetal malformations, but also prenatally determine the nosology of this disease. In other words, the diagnosis was not 'multiple birth defects' if it is not possible to talk about the etiology of the disease and therefore about specific preventive measures for this pathology later in the family. The prenatal diagnosis was complete and correct, which is particularly important for genetic counseling and the development of appropriate reproductive behavior in the family in the future.

Since there are few publications on the prenatal diagnosis of this genetic syndrome, we present a series of our own observations on the diagnosis of EEC syndrome at different stages of pregnancy, including the first trimester. Special considerations are made on the specificity of genetic counseling in the diagnosis of the different forms of the syndrome with autosomal dominant inheritance type, with the aim of developing specific preventive measures for this inherited disease.

symptoms of the disease

The clinical picture of ectrodactyly of the limbs is very variable. According to the classification, two forms of ectrodactyly are distinguished:

1. Primary. Depending on the severity, symptoms include underdevelopment of the nail phalanges (mild), nail and middle phalanges (moderate), nail, middle and large phalanges (severe).
2. Secondary. Combined with shortening of the fingers due to underdevelopment of the middle and main phalanges (brachydactyly), partial or complete fusion of all fingers or toes (syndactyly), or a change in the shape or deviation of the fingers.

To explain what ectrodactyly is, it is enough to list the main symptoms of this condition:

Based on the number of deformed fingers, monodactyly (e.g. absence of the big toe), di-, tri- and tetradactyly (defect of 2, 3 or 4 fingers, respectively) can be diagnosed. The splitting of the fingers into two parts and the formation of a cone-shaped gap between the fingers is called finger hand and is usually hereditary.

Diagnosis of ectrodactyly

Abnormal development of the fingers and toes can be diagnosed during an ultrasound scan during pregnancy. If an anomaly such as ectrodactyly is detected, your doctor will tell you during the consultation what anomaly it is and what recommendations you should follow. If it is determined that the anomaly is inherited in an autosomal dominant manner, abortion is recommended.

If a condition such as ectrodactyly is diagnosed, molecular genetic testing can determine the cause of the lesion if it is a change in the genome. If the disease is diagnosed at birth, a radiological examination is carried out to determine the type of pathology and determine further treatment tactics.

Advice and recommendations from an orthopedist at Perseus Orthopedic Center. Tel. 8 (495) 469-99-05

• Orthopedic Surgery
  • Consultation with a podiatrist
  • online consultation
  • Visit a specialist
  • Manufacture of footwear
  • Orthotics, traction insoles
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  • Stabilization in cerebral palsy
  • Insoles for clubfoot.
  • With reinforced arch support
  • For dynamic splints
  • For children
  • teenagers
  • Adult
  • Home
  • Supportive footwear for prostheses and aids
  • For diabetics
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  • Custom made insoles

  Orthopedic shoes that are mounted on standard lasts
  Shoes with insoles that can be shortened up to 3 cm
  The type of footwear is selected by the podiatrist at the appointment and depends on the functional condition and individual anatomy of the patient's feet.

  2. complex orthopedic shoes according to impression and outline

  3. Low shoes with torso compensation (bevel towards the arch of the foot) 4.

  4. Clip with adjustable shortening 4.

  4а. Insole to compensate for shortening across the entire range of motion
  4b. Insole with partial shortening compensation along the heel + bevel under the heel.

  5. the shortening compensation along the sole including the patient's shoe.

  6. Half-sided insole with shortening compensation (diagonally under the heel) + shortening compensation along the sole 8.

  8. Massage, manual therapy
  Schedule an appointment

  The design depends on the degree of shortening and is determined by the podiatrist during the consultation

  Compensation for limb shortening

  Different limb lengths in the same person have different origins. Shortening of a limb can be congenital, it can develop as a result of trauma when a bone fracture has not healed properly, it can be caused by disease of the bone growth zones causing them to close, or by disease of a joint causing a flexion contracture caused. Shortening can affect every single bone in the lower limbs and have a negative impact on overall length. The shortening of the leg impairs walking. A small difference in length can easily be compensated for by the patient without orthopedic help. Larger differences in length between limbs are usually corrected either by an orthosis or cancellation or by plantar lengthening of the sole. The orthopedist decides whether to compensate for the shortening based on the patient's diagnosis.

  Hemihypoplasia, congenital hip dislocation, club foot, proximal femoral varus, dyschondroplasia, multiple exostoses

  Damage to the epiphyseal plate, disruption of the femoral or tibial growth zone in osteitis, tuberculosis, arthritis

  Femoral or tibial diaphyritis, Brody abscess, metaphyseal tuberculosis, arthritis, syphilis, elephantiasis, venous thrombosis

  Hemangioma, lymphangioma, giant cell tumor, fibrous osteitis, neurofibromatosis, fibrous dysplasia

  1. Shortening compensation on the sole of the shoe, also on the patient's shoe

  

  

  3. Compensation for shortening in combination

  Forms of congenital hand cleft in children

  The congenital hand cleft is divided into several forms depending on the severity of the anomaly:

  1. Simple shape. It is characterized by a deformity of the hand with the absence of one or two central rays and fingers. A large gap appears between the fingers that looks like a crack. Sometimes it extends to the carpal bones.
  2. Complex shape. The hand deformity is also characterized by the absence of one or two rays and fingers, but is accompanied by other malformations. Another characteristic feature is the presence of a transverse phalanx.
  3. Atypical form. In most cases, the atypical form includes malformations in which only the 4th and 5th fingers are preserved. The transverse phalanx is always present. Such malformations can be classified as the most serious form. They are almost always accompanied by a cleft foot. In addition, other types of anomalies may also be present (cleft lip, clubfoot, etc.). The atypical form results in severe functional impairments and the absence of the hand.

  The development of a cleft hand in children is usually due to hereditary factors. The defect is inherited in an autosomal dominant manner, i.e. from parent to child. Gene mutations are occasionally observed in which the abnormality does not affect the recipients but is passed down through one or two generations.

  Clinical manifestations and diagnosis

  Congenital hand clefts in children are accompanied by the following symptoms

  • absence of one or more middle fingers;
  • Significant limitation or complete absence of grasping function;
  • The hand has a deformed appearance (known as ugly hand deformity);
  • The preserved fingers are often fused;
  • contractures of the interphalangeal joints;
  • Clinodactyly (curvature of the fingers).

  Due to impaired functionality, children with this anomaly often have great difficulty performing simple tasks, including self-care. Cosmetic abnormalities often lead to communication and learning difficulties.

  Radiological examination is the most important method for diagnosing cleft hand. The results of the X-ray examination are sufficient to determine the treatment regimen. In addition, various clinical examinations may be carried out, particularly in preparation for surgery. The purpose of the medical examination is to identify any contraindications to surgery.

  Advice and recommendations from an orthopedic surgeon at the Perseus Orthopedic Center. Tel. 8 (495) 469-99-05

  • Orthopedic practice
    • Advice from a podiatrist
    • online consultation
    • Visit a specialist
    • Manufacture of footwear
    • orthoses, traction insoles
    • Orthoses and braces for the lower limbs
    • Stabilization in cerebral palsy
    • Varus insoles for clubfoot.
    • With reinforced arch support
    • For dynamic splints
    • For children
    • teenager
    • Adult
    • Home
    • Supportive footwear for prostheses and aids
    • For diabetics
    • Tactical shoes, military shoes, outdoor and hiking shoes
    • Custom insoles

    Clubfoot treatment with orthopedic footwear. Treatment of club feet. Perseus. 12

    

    2 Complicated orthopedic footwear made from impression and contouring

    Shoes with inserted tunic, stiff buttocks and lengthened ankle boots, lengthened heels: P-45, P-48, P-14

    

    

    Equinovarus Ankle Leather Corset

    The splint is designed to accommodate hard-soled orthopedic shoes.

    

    Clubfoot in children

    

    Clubfoot is a deformity of the distal part of the lower limbs.
    It includes equinus, varus, cavus and anterior adduction. Club feet are more common in boys than girls.
    Infantile clubfoot is congenital. In newborns, this defect occurs with a frequency of one in a thousand. Acquired clubfoot occurs as a result of nerve disease or trauma.
    Massage and incremental affusions are required for effective treatment of clubfoot.
    The best treatment results are achieved when treatment is started in infancy. Success depends on age and the severity of the deformity. Orthopedic shoes are prescribed for minor deformities when a cast is not indicated, or after cast removal to prevent recurrence of the deformity. During the child's active growth, the foot deformity may recur. If treatment is started on time, the prognosis is favorable. Clubfoot is a developmental disorder that can be identified soon after the baby is born. In the early stages, clubfoot in children can be treated in a short time. If clubfoot is neglected or not properly treated beforehand, children can lose their ability to walk normally and become disabled. If you suspect this condition in your child, you should immediately contact an orthopedist who can make the correct diagnosis.

    In our center you will receive the necessary advice on clubfoot treatment and can confirm or allay your fears. It takes about two months to correct this anomaly in a child. The main factor that can delay clubfoot treatment for a long period of time is the severity of the deformity. In this case, surgical intervention can be avoided. Chiropractic adjustments, orthopedic shoes, and a gradual change in casts can help. Clubfoot is a developmental disorder that can be diagnosed at birth. In the early stages, clubfoot in infants can be treated in a short time. If clubfoot is neglected or not treated properly, children can lose their normal ability to move and become disabled. If you suspect this condition in your child, you should immediately contact an orthopedist who can make the correct diagnosis. At the Perseus Orthopedic Center you will receive the advice you need to prevent and treat clubfoot. If treatment begins promptly, the prognosis is favorable.

    Syndactyly

    Syndactyly – toes that grow together – occurs in both girls and boys. It can be unilateral or bilateral and can occur on both hands and feet. Syndactyly of the hands is usually sought because it is the predominant cosmetic defect.

    The most common form is the cutaneous form, in which the fingers have all the anatomical structures but are connected by the skin, and less often the bony form, in which the bones of the phalanges of the fingers are fused together (more often the distal phalanges). Fusion usually occurs between fingers II-III or IV-V.
    Initially the function of the toes is not affected, but as the toes grow there may be a difference in length between the fused (bony) toes, with the longer toe permanently slightly bent at the middle interphalangeal joint. In syndactyly of the foot, the toes are normally developed.

    The type of syndactyly is clarified by an X-ray examination.

    Treatment Exclusive surgical separation of the toes. The technically simplest operation is toe separation in patients with ligamentous syndactyly. The ligaments are straightened or excised and the wounds are stitched up.

    Cutaneous syndactyly requires special preparation: stretching the skin so that the surgical wounds on each finger can be sutured without tension and healing takes place with the original tension. If the skin is insufficiently stretched, the tension leads to tissue necrosis or spreading of the edges of the wound, and the rough scar formed during growth leads to a lateral curvature of the finger. Therefore, parents are recommended to massage and stretch the fingers for a month, and only then, when the skin reserve is created, the patient undergoes surgery. An original method of preparation was proposed by AN Gudushauri and LA Tvaliashvili: stretching the fingers with a distraction device.

    After preparation, the fingers are separated using the Dido method so that the seams between the fingers do not overlap. To avoid scarring at the base of the finger, the interdigital fold should be shaped using the Celler method (triangular flap) or with two opposing triangular skin flaps that overlap the interdigital space. If there is a skin defect, a free primary autodermoplasty is performed.

    Polydactyly

    This is an enlarged finger that occurs primarily on the hands and can be unilateral or bilateral. The number of additional fingers varies, including on both sides of the hand. Polydactyly may present as underdeveloped distal phalanges on a thin stalk or as anatomically normal extra fingers with extra metacarpals.

    The diagnosis is clarified radiologically. The patient is treated like a cosmetic defect. The operation consists of removing the supernumerary fingers and supernumerary metacarpals and restoring the shape of the hand.

    Defects causing shortening of the lower limbs

    Defects that shorten the lower limbs (ectromelia) – is a congenital anomaly of limb development associated with severe underdevelopment or absence of limbs [1].

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    classification

    Clinical classification of lower limb shortening defects (ectromelia) [1].

    Types of lower limb shortening defects (ectromelia):

    • Distal ectromelia (distal ectromelia) – is an anomaly affecting the distal part of the limbs.
    There are the following types of distal ectromelia:
    – Type of the first toe. – Hypoplasia or aplasia of the thumb or the presence of a three-toed thumb;
    – tibia type – Hypoplasia or aplasia (partial or complete) of the tibia. It is associated with shortening and inward curvature of the tibia, malformation of the fibula, foot, underdevelopment of the quadriceps femoris muscle and abnormalities of the kneecap. Abnormalities of the lower leg and foot muscles were also observed. The deformity is often accompanied by aplasia of the I-II metatarsals and the corresponding toes, and sometimes by fibular duplication. It occurs more often on one side and less often on both sides.

    • Axial ectromelia – Absence or hypoplasia of both distal and proximal limbs.
    Depending on the extent of the process, a distinction is made:
    – long axial type Leg – hypoplasia or partial aplasia of the thigh with partial aplasia of the tibia or complete aplasia of the tibia;
    – intermediate type Leg - subtotal femoral aplasia with partial or total tibial aplasia;
    – Short axial type Leg – subtotal femoral aplasia with partial or complete tibial aplasia.

    • Proximal ectromelia – A defect in the proximal part of the leg (femur) without damage to the distal part. It manifests itself in a shortening and curvature of the femur; the kneecap and fibula may be missing.

    The following types are distinguished:
    – Long proximal type. – Hypoplasia of the femur or partial aplasia of the femur;

    – Intermediate proximal type – Subtotal femoral aplasia;
    – short proximal type – total hip aplasia.

    Dental problems in a child

    Sometimes parents come to us complaining that their child's teeth in the lower or upper jaw are missing or have not erupted even though they should erupt at the appropriate age. In such cases, an experienced pediatrician will have an x-ray taken. This x-ray can be used to determine whether a tooth is present or not. If there is no tooth at all, we refer the child to an endocrinologist. He or she will administer treatment and find out the cause of the disease. A temporary denture can then be inserted.

    Supernumerary teeth are removed whenever they get in the way. Supernumerary teeth are usually impacted teeth, meaning teeth that have not erupted and remain in the gums. In such cases they must also be pulled.

    Obligatory specialist medical advice

    Children with congenital jaw malformations and their parents should be examined by a geneticist. For what purpose? The geneticist must say what the cause of the disease is, how it arose. The geneticist must develop a treatment plan so that the defect is not passed on to the next child after birth. The geneticist can advise at what age, when and at what point in time you should father another child. He is a specialist who not only treats genetics but also observes the children. It is mandatory that both the parents and the child with congenital anomalies are observed by geneticists.

    

    Finally, I would like to address the parents. Our medical center 'Healer' in Makhachkale has a very good, modern department with experienced and highly qualified doctors. We eliminate and treat all types of developmental disorders, malformations of the jaw-facial area (facial asymmetry, cosmetic defects) in children in Dagestan, and also recommend and carry out measures for the child's subsequent social adaptation. You don't have to travel outside the country. All operations of any complexity performed by specialists in Moscow and other cities of central Russia are successfully carried out here. Please contact us, we will be happy to help you. Take good care of yourself and your children!

    Zhakhbarov Akhmed Gamzatovich, surgeon, maxillofacial surgeon in Makhachkala.

• longitudinal ectromelia.
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• Ehlers-Danlo Syndrome.